|
|
|
|
|
|
|
||||
| Home Help Feedback Subscriptions Archive Search Table of Contents | ||||
Development, Vol 125, Issue 4 557-566, Copyright © 1998 by Company of Biologists
JOURNAL ARTICLES |
T Self, M Mahony, J Fleming, J Walsh, SD Brown and KP Steel
MRC Institute of Hearing Research, University Park, Nottingham NG7 2RD, UK.
The mouse shaker-1 locus, Myo7a, encodes myosin VIIA and mutations in the orthologous gene in humans cause Usher syndrome type 1B or non-syndromic deafness. Myo7a is expressed very early in sensory hair cell development in the inner ear. We describe the effects of three mutations on cochlear hair cell development and function. In the Myo7a816SB and Myo7a6J mutants, stereocilia grow and form rows of graded heights as normal, but the bundles become progressively more disorganised. Most of these mutants show no gross electrophysiological responses, but some did show evidence of hair cell depolarisation despite the disorganisation of their bundles. In contrast, the original shaker-1 mutants, Myo7ash1, had normal early development of stereocilia bundles, but still showed abnormal cochlear responses. These findings suggest that myosin VIIA is required for normal stereocilia bundle organisation and has a role in the function of cochlear hair cells.
This article has been cited by other articles:
|
|
 |
|
  H. M. Prosser, A. K. Rzadzinska, K. P. Steel, and A. Bradley Mosaic Complementation Demonstrates a Regulatory Role for Myosin VIIa in Actin Dynamics of Stereocilia Mol. Cell. Biol., March 1, 2008; 28(5): 1702 - 1712. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Etournay, I. Zwaenepoel, I. Perfettini, P. Legrain, C. Petit, and A. El-Amraoui Shroom2, a myosin-VIIa- and actin-binding protein, directly interacts with ZO-1 at tight junctions J. Cell Sci., August 15, 2007; 120(16): 2838 - 2850. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Chung, S. Kim, J. Yoon, P. N. Adler, and J. Yim The Balance Between the Novel Protein Target of Wingless and the Drosophila Rho-Associated Kinase Pathway Regulates Planar Cell Polarity in the Drosophila Wing Genetics, June 1, 2007; 176(2): 891 - 903. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. R. Phillips, S. Tong, R. Goodyear, G. P. Richardson, and J. L. Cyr Stereociliary Myosin-1c Receptors Are Sensitive to Calcium Chelation and Absent from Cadherin 23 Mutant Mice. J. Neurosci., October 18, 2006; 26(42): 10777 - 10788. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Kremer, E. van Wijk, T. Marker, U. Wolfrum, and R. Roepman Usher syndrome: molecular links of pathogenesis, proteins and pathways Hum. Mol. Genet., October 15, 2006; 15(suppl_2): R262 - R270. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. H. Gagnon, C. M. Longo-Guess, M. Berryman, J.-B. Shin, K. W. Saylor, H. Yu, P. G. Gillespie, and K. R. Johnson The Chloride Intracellular Channel Protein CLIC5 Is Expressed at High Levels in Hair Cell Stereocilia and Is Essential for Normal Inner Ear Function J. Neurosci., October 4, 2006; 26(40): 10188 - 10198. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Yang, M. Kovacs, T. Sakamoto, F. Zhang, D. P. Kiehart, and J. R. Sellers Dimerized Drosophila myosin VIIa: A processive motor PNAS, April 11, 2006; 103(15): 5746 - 5751. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Watanabe, R. Ikebe, and M. Ikebe Drosophila Myosin VIIA Is a High Duty Ratio Motor with a Unique Kinetic Mechanism J. Biol. Chem., March 17, 2006; 281(11): 7151 - 7160. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Senften, M. Schwander, P. Kazmierczak, C. Lillo, J.-B. Shin, T. Hasson, G. S. G. Geleoc, P. G. Gillespie, D. Williams, J. R. Holt, et al. Physical and Functional Interaction between Protocadherin 15 and Myosin VIIa in Mechanosensory Hair Cells J. Neurosci., February 15, 2006; 26(7): 2060 - 2071. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. El-Amraoui and C. Petit Usher I syndrome: unravelling the mechanisms that underlie the cohesion of the growing hair bundle in inner ear sensory cells J. Cell Sci., October 15, 2005; 118(20): 4593 - 4603. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. M. G. Smits, T. A. Peters, J. D. Mul, H. J. Croes, J. A. M. Fransen, A. J. Beynon, V. Guryev, R. H. A. Plasterk, and E. Cuppen Identification of a Rat Model for Usher Syndrome Type 1B by N-Ethyl-N-nitrosourea Mutagenesis-Driven Forward Genetics Genetics, August 1, 2005; 170(4): 1887 - 1896. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. I. Tuxworth, S. Stephens, Z. C. Ryan, and M. A. Titus Identification of a Myosin VII-Talin Complex J. Biol. Chem., July 15, 2005; 280(28): 26557 - 26564. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. M. Longo-Guess, L. H. Gagnon, S. A. Cook, J. Wu, Q. Y. Zheng, and K. R. Johnson A missense mutation in the previously undescribed gene Tmhs underlies deafness in hurry-scurry (hscy) mice PNAS, May 31, 2005; 102(22): 7894 - 7899. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Delprat, V. Michel, R. Goodyear, Y. Yamasaki, N. Michalski, A. El-Amraoui, I. Perfettini, P. Legrain, G. Richardson, J.-P. Hardelin, et al. Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly Hum. Mol. Genet., February 1, 2005; 14(3): 401 - 410. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Adato, V. Michel, Y. Kikkawa, J. Reiners, K. N. Alagramam, D. Weil, H. Yonekawa, U. Wolfrum, A. El-Amraoui, and C. Petit Interactions in the network of Usher syndrome type 1 proteins Hum. Mol. Genet., February 1, 2005; 14(3): 347 - 356. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S.-i. Kitajiri, K. Fukumoto, M. Hata, H. Sasaki, T. Katsuno, T. Nakagawa, J. Ito, S. Tsukita, and S. Tsukita Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia J. Cell Biol., August 16, 2004; 166(4): 559 - 570. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. E. Futter, J. S. Ramalho, G. B. Jaissle, M. W. Seeliger, and M. C. Seabra The Role of Rab27a in the Regulation of Melanosome Distribution within Retinal Pigment Epithelial Cells Mol. Biol. Cell, May 1, 2004; 15(5): 2264 - 2275. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. R. Johnson, L. H. Gagnon, L. S. Webb, L. L. Peters, N. L. Hawes, B. Chang, and Q. Y. Zheng Mouse models of USH1C and DFNB18: phenotypic and molecular analyses of two new spontaneous mutations of the Ush1c gene Hum. Mol. Genet., December 1, 2003; 12(23): 3075 - 3086. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. J. Karolyi, F. J. Probst, L. Beyer, H. Odeh, G. Dootz, K. B. Cha, D. M. Martin, K. B. Avraham, D. Kohrman, D. F. Dolan, et al. Myo15 function is distinct from Myo6, Myo7a and pirouette genes in development of cochlear stereocilia Hum. Mol. Genet., November 1, 2003; 12(21): 2797 - 2805. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. J. Goodyear, P. K. Legan, M. B. Wright, W. Marcotti, A. Oganesian, S. A. Coats, C. J. Booth, C. J. Kros, R. A. Seifert, D. F. Bowen-Pope, et al. A Receptor-Like Inositol Lipid Phosphatase Is Required for the Maturation of Developing Cochlear Hair Bundles J. Neurosci., October 8, 2003; 23(27): 9208 - 9219. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Dabdoub, M. J. Donohue, A. Brennan, V. Wolf, M. Montcouquiol, D. A. Sassoon, J.-C. Hseih, J. S. Rubin, P. C. Salinas, and M. W. Kelley Wnt signaling mediates reorientation of outer hair cell stereociliary bundles in the mammalian cochlea Development, June 1, 2003; 130(11): 2375 - 2384. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y. Kikkawa, H. Shitara, S. Wakana, Y. Kohara, T. Takada, M. Okamoto, C. Taya, K. Kamiya, Y. Yoshikawa, H. Tokano, et al. Mutations in a new scaffold protein Sans cause deafness in Jackson shaker mice Hum. Mol. Genet., March 1, 2003; 12(5): 453 - 461. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Weil, A. El-Amraoui, S. Masmoudi, M. Mustapha, Y. Kikkawa, S. Laine, S. Delmaghani, A. Adato, S. Nadifi, Z. B. Zina, et al. Usher syndrome type I G (USH1G) is caused by mutations in the gene encoding SANS, a protein that associates with the USH1C protein, harmonin Hum. Mol. Genet., March 1, 2003; 12(5): 463 - 471. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. P. Steel Varitint-waddler: A double whammy for hearing PNAS, November 12, 2002; 99(23): 14613 - 14615. [Full Text] [PDF]
|
|
|
|
|
|
J. Siemens, P. Kazmierczak, A. Reynolds, M. Sticker, A. Littlewood-Evans, and U. Muller The Usher syndrome proteins cadherin 23 and harmonin form a complex by means of PDZ-domain interactions PNAS, November 12, 2002; 99(23): 14946 - 14951. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Forge and T. Wright The molecular architecture of the inner ear Br. Med. Bull., October 1, 2002; 63(1): 5 - 24. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. L. Mitchem, E. Hibbard, L. A. Beyer, K. Bosom, G. A. Dootz, D. F. Dolan, K. R. Johnson, Y. Raphael, and D. C. Kohrman Mutation of the novel gene Tmie results in sensory cell defects in the inner ear of spinner, a mouse model of human hearing loss DFNB6 Hum. Mol. Genet., August 1, 2002; 11(16): 1887 - 1898. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Van Aelst and M. Symons Role of Rho family GTPases in epithelial morphogenesis Genes & Dev., May 1, 2002; 16(9): 1032 - 1054. [Full Text] [PDF]
|
|
|
|
|
|
I. P. Udovichenko, D. Gibbs, and D. S. Williams Actin-based motor properties of native myosin VIIa J. Cell Sci., January 15, 2002; 115(2): 445 - 450. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Tsai, R. E. Hardisty, C. Rhodes, A. E. Kiernan, P. Roby, Z. Tymowska-Lalanne, P. Mburu, S. Rastan, A. J. Hunter, S. D. M. Brown, et al. The mouse slalom mutant demonstrates a role for Jagged1 in neuroepithelial patterning in the organ of Corti Hum. Mol. Genet., March 1, 2001; 10(5): 507 - 512. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. T. Libby and K. P. Steel Electroretinographic Anomalies in Mice with Mutations in Myo7a, the Gene Involved in Human Usher Syndrome Type 1B Invest. Ophthalmol. Vis. Sci., March 1, 2001; 42(3): 770 - 778. [Abstract] [Full Text]
|
|
|
|
|
|
S. Ernest, G.-J. Rauch, P. Haffter, R. Geisler, C. Petit, and T. Nicolson Mariner is defective in myosin VIIA: a zebrafish model for human hereditary deafness Hum. Mol. Genet., September 1, 2000; 9(14): 2189 - 2196. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. W. Anderson, F. J. Probst, I. A. Belyantseva, R. A. Fridell, L. Beyer, D. M. Martin, D. Wu, B. Kachar, T. B. Friedman, Y. Raphael, et al. The motor and tail regions of myosin XV are critical for normal structure and function of auditory and vestibular hair cells Hum. Mol. Genet., July 22, 2000; 9(12): 1729 - 1738. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. P Steel Science, medicine, and the future: New interventions in hearing impairment BMJ, March 4, 2000; 320(7235): 622 - 625. [Full Text]
|
|
|
|
|
|
M. Ashburner, S. Misra, J. Roote, S. E. Lewis, R. Blazej, T. Davis, C. Doyle, R. Galle, R. George, N. Harris, et al. An Exploration of the Sequence of a 2.9-Mb Region of the Genome of Drosophila melanogaster: The Adh Region Genetics, September 1, 1999; 153(1): 179 - 219. [Abstract] [Full Text]
|
|
|
|
|
|
B. Riley, M Chiang, L Farmer, and R Heck The deltaA gene of zebrafish mediates lateral inhibition of hair cells in the inner ear and is regulated by pax2.1 Development, January 12, 1999; 126(24): 5669 - 5678. [Abstract] [PDF]
|
|
|
|
 |
|
 F. J. Probst, R. A. Fridell, Y. Raphael, T. L. Saunders, A. Wang, Y. Liang, R. J. Morell, J. W. Touchman, R. H. Lyons, K. Noben-Trauth, et al. Correction of Deafness in shaker-2 Mice by an Unconventional Myosin in a BAC Transgene Science, May 29, 1998; 280(5368): 1444 - 1447. [Abstract] [Full Text]
|
|
|
|
|
|
P. Kussel-Andermann, A. El-Amraoui, S. Safieddine, J.-P. Hardelin, S. Nouaille, J. Camonis, and C. Petit Unconventional Myosin VIIA Is a Novel A-kinase-anchoring Protein J. Biol. Chem., September 15, 2000; 275(38): 29654 - 29659. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. E. Kiernan, N. Ahituv, H. Fuchs, R. Balling, K. B. Avraham, K. P. Steel, and M. Hrabe de Angelis The Notch ligand Jagged1 is required for inner ear sensory development PNAS, March 27, 2001; 98(7): 3873 - 3878. [Abstract] [Full Text] [PDF]
|
|
